颈部淋巴结边缘区淋巴瘤累及脾脏并伴显著肉芽肿反应病理分析

Clinicopathological Features of the Nodal Marginal Zone B-cell Lymphoma Involving Spleen Combined with Prominent Granulomatous Reaction

目的探讨颈部淋巴结边缘区淋巴瘤(nodal marginal zone lymphoma,NMZL)累及脾脏并伴显著肉芽肿反应的临床病理学特征和鉴别诊断要点。方法回顾性分析1例颈部NMZL累及脾脏并伴显著肉芽肿反应临床资料,分析其病理组织学和免疫表型、基因检测特征。结果男,34岁,因低热、消瘦、盗汗16个月,全身淋巴结及脾脏增大14个月就诊,初步诊断为脾功能亢进行脾脏切除术。脾脏活检及免疫组织化学染色(免疫组化)示:脾脏组织内多个结节样结构,周边见较多小中淋巴样细胞与组织细胞混合存在,部分结节间见较多淋巴样细胞,小中淋巴样细胞浸润红髓。脾脏内结节区CD68和CD163阳性表达,结节周围有较多CD20阳性的小中淋巴样细胞,部分结节间区淋巴样细胞聚集融合,Ki-67阳性率30%。倾向低级别B细胞淋巴瘤。右颈部淋巴结活检示:淋巴结仅见少量残存淋巴滤泡,小中淋巴细胞弥散分布。免疫组化示:淋巴结内小中淋巴样细胞CD20、CD43阳性,Bcl-2(部分阳性),Ki-67阳性率约40%。淋巴结基因检测发现克隆性基因重排。最终诊断:右颈部NMZL累及脾脏并伴显著肉芽肿反应。结论肉芽肿病变可能掩盖淋巴瘤的形态学改变,临床病理诊断时除需注重特殊染色排除特殊感染或其他病因,还需仔细分析肉芽肿之间的淋巴细胞,综合临床、形态学、免疫组化及分子遗传学检查结果以排除淋巴瘤的可能。

Objective To investigate the clinical and pathological features of nodal marginal zone B-cell lymphoma involving spleen combined with prominent granulomaous reaction. Methods The histopathological features, immunopheno- type and gene rearrangement characteristics were utilized to analyze one case of nodal marginal zone B-cell lymphoma involving spleen with combined prominent granulomatous reaction retrospectively. Results This patient was a male, 34 years old. The spleen was resected due to hypersplenism accompanied with fever, weight loss, night sweat for 16 months, systemic lymph node and spleen enlarged for 14 months. Under the microscope and immunohistochemical staining： There are many nodules distributed in the spleen tissue, surround by small-medium size lymphocytes and histocytes, and the lymphocytes distributed between some nodules and even invade red pulp. The nodules of spleen expressed CD68, CD163 but not CD10, CD21. The small-medium size lymphocytes around the nodules were CD20 positive, and the Ki-67 index was about 30% . Low grade B cell lymphoma was supposed. Right neck lymph node showed lymph node structure was destroyed by diffusely distributed small-medium size lymphocytes with only a small amount of residual lymph follicles. The immunohistochemical staining showed the small-medium size lymphocytes were CD20, CD43 positive, Bcl-2 partial positive, and the Ki-67 index was about 40% . Clonal rearrangement detected by lymph node gene detection. Diagnosis ： marginal zone B cell lymphoma of right neck lymph node, and involve spleen with combined prominent granulomatous reaction. Conclusion Besides exclusion of specific infec-tions of the lymph node granulomas, the possibility of lymphoma should be ruled out by carefully analysis of clinical, morpho-logical ,immunohistochemical and molecular genetics characteristics.